Abstract:Immunodeficiency disorders result in a full or partial impairment of the immune
system. We can divide them into primary and secondary immunodeficiency.
Primary disorders of humoral immunity are th...Immunodeficiency disorders result in a full or partial impairment of the immune
system. We can divide them into primary and secondary immunodeficiency.
Primary disorders of humoral immunity are the most common type of primary
immunodeficiency. Typical representatives are: selective IgA deficiency, Bruton
agammaglobulinemia, CVID and Hyper IgM syndrome.
Patients with cellular immunity defects are in more severe medical conditions. Typical
of this group is DiGeorge's syndrome.
The most severe form of immunodeficiency are complex disorders of humoral and cellular
immunity such as: SCID, CID, Wiskott-Aldriche syndrome, IPEX syndrome, ALPS, ataxiateleangiectasia
syndrome. In primary disorders of non-specific immunity, we can mention
hyper IgE syndrome, chronic granulomatous disease, and complement disorders.
The most common causes of secondary immunodeficiency are malnutrition, the HIV
infection and treatment of malignant diseases with cytostatic and radiation and the loss of
protein in nephrotic syndrome and enteropathies.
Patients with immune deficiency have frequent and recurent infections often with
unusual infectious agents. In infancy immunodeficiency can lead to child death if the disease
is not recognised on time and adequately treated. For this reason, it would be good to
introduce neonatal screening for SCID, as it is used for screening in the USA.
Treatment of primary disorders of humoral immunity is carried out by regular
administration of IVIG and treatment of bacterial infections with antibiotics. In cellular and
combined immunodeficiency, it is necessary to perform transplantation of hematopoetic stem
cells or in some cases perform gene therapy as soon as possible. If the diagnosis is made in
the neonatal period or within the first 3 months of life the treatment success is greater than
90 %, otherwise it can lead to death.Read More
Publication Year: 2020
Publication Date: 2020-01-01
Language: en
Type: dissertation
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